| Author | Message | ||
     GFM |
On June 30, my 2-day old daughter was diagnosed with high-risk LQT. The doctors say she’s high-rish because of her LQT (600+), early presentation age and 2:1 AV Heart Block. Since her pacemaker implantation (July 1), beta-blocker and mexiletine medicine treatments, her LQT has decreased, now ranging from 440 to 480, her heart block isn't present anymore, her avg. heart rate is 127 and she’s only pacing 6%. Her genetic tests revealed that she has LQT 3 (sodium channel). My wife and I are trying to learn and research about Long QT and treatments. As her parents, we want to provide every possible protection so our daughter has an opportunity to make her goals and dreams come true. We live in Pennsylvania (near Philadelphia) and we definitely plan on continuing treatment with her EP. However, we’re interested in other’s opinions and specifically about the work others are doing with ICDs for infants. We were wondering how many newborn/infant LQT patients have been treated, since we understand diagnosing LQT at this age is extremely rare? Can you tell us under what circumstances should an ICD be considered for an infant? What are the risks? Are there reasons to wait for a child in the high-risk LQT category to get bigger? Is this something currently being researched? How often do ICDs in infants have to be changed? Her hospital sent us home with an AED and a heart monitor (sleep & car rides) for added protection. We're currently getting a customized AED for our daughter. However, we would feel better knowing that any intervention that needed to occur would be instantaneous. Thanks for your input! | ||
| Dr. Jorgen Kanters Username: Jorgen Registered: 08-2003 |
ICD treatment in the newborn and infant is very difficult. Due to the size of the ICD it has to place in the abdomen and the electrodes placed on the surface of the heart. This a little risky and most centers will wait until an older age before implanting an ICD. Our youngest LQTS patient with an ICD was 9 years at implantation. However we have in Denmark put an ICD in a 6 month kid due to repetitive arrhythmias (not LQTS) | ||
| GFM |
Dr. Kanters: Thanks for the response! We live in Philadelphia, Pennsylvania and are currently treating at Children's Hospital of Philadelphia. I've already received some ICD criteria feedback from Children's Hospital of Boston. Although my daughter seems to be currently doing well, we're very concerned about the ambiguity of her LQT3. Our EP said that our daughter might eventually be a candidate for an ICD because of her LQT3 diagnosis. As her parents, our biggest concern is how long we can safely wait! Once I receive genetic test results from my immediate family (wife, son and me), I would like to seek additional consultations with other EPs around the world. In your perspective, can EPs provide a good analysis by only seeing the patient's records? If so, what kind of information should I gather beyond the genetic test results? She recently had a 24-hour holter monitor test. Are the LQT readings throughout the different times of the day important for me to track? Thank you for your time, efforts & dedication to help families trying to learn and live during all of our LQT Adventures! (-: | ||
| Dr. Jorgen Kanters Username: Jorgen Registered: 08-2003 |
LQT3 is rare and it seems that betablockers are not very effective. Mexilitine shortens the QT interval but no one knows if it reduces mortality. ICD implantation is not without risk especially in infants or small children. There is no definite answer to your questions. WE probably would implant an Icd at a later stage when the kid has grown older, but it had to be individual evaluated | ||
| Jmac |
Glenn: I love this website and use it daily. Dr. Kanters is super, always there for everybody's questions, don't know how he does it, thanks Doc! Anyway, there is another website that is also super many LQT patients on there and much support from each other. Many have small children and even infants with ICD's, would be good to chat with, the emotions can run high, but so good to know your diagnosis and what you are dealing with than not to know! Go to www.longqt@yahoogroups.com You will have to register but it is free. Best of luck with little one. Jmac | ||
| GFM |
Dr. Kanters: It seems like my daughter is responding well courtesy of her growth & development combined with the betablockers and Mexilitine. She's now 12+ lbs. and keeps gaining weight. We'll continue keeping close tabs on her status and can hopefully safely wait until she's older for an ICD. Do you have many LQT3 patients? How about any LQT newborn patients? It amazes me how infrequently the EPs have seen LQT newborn patients at two top Children's Hospitals in the USA (Philadelphia & Boston)! Both hospitals hadn't seen a LQT newborn in 2 years. I'm trying to find a doctor somewhere in the world doing extensive research on LQT newborns? I've heard there just isn't a large enough pool of these type of LQT patients. Thanks for your responses and insight. It's fantastic that doctors like yourself make the effort to educate families about the LQT Journey! | ||
| GFM |
JMAC: I agree about this informative website. Although I've only been in the LQT Club for 4-months, I've thoroughly appreciated reading all of Dr. Kanter's responses during my LQT cram course! Thanks for the tip on the longqt yahoo group. In July, I joined the group and learned so much from the perspectives of many LQT people and family members battling LQT everyday. It's been so helpful to correspond with others who know exactly what the LQT Lifestyle is like. I've even communicated via e-mail and phone with a few members of newborns and small children. So far, our LQT Adventure has been some rollercoaster ride. Have a great day! GMF | ||
| jmac |
Glenn: Ever since LQT, life has become just that a roller coaster ride, ups and down, but mostly ups because of places like these websites. At first I listened mostly trying to grasp the reality of it all.. how can this be? all of those questions just like you guys. We are so fortunate to know that we have this and learn how to live with it...not to have to find out the hard way...after someone you love died from it..Keep chatting. We are from Louisiana, where are you guys from? Jmac | ||
| GFM |
Jmac: My family lives in the Philadelphia area and we're currently treating at Children's Hospital of Philadelphia. How long has your family been part of the LQT Game? Have you lost a family member? Does more than one person in your family have LQT? We're awaiting genetic test results from my immediate family (wife, son and me) and hoping that our daughter is the only LQT member. LQT Knowledge has helped us be more prepared for our family's adventure to love, learn & laugh! Hopefully your family is overcoming any obstacles caused by Katrina! Everything OK? Keep Smiling (-: GMF | ||
| Dr. Jorgen Kanters Username: Jorgen Registered: 08-2003 |
LQTS in newborn is rare entity. I am not aware of any doing research specific on that. We have a couple of newborns here, but none of LQT3. However your kid is treated in Philadelphia just like I would have done it, so I believe your are in the best hands possible | ||
| Jmac |
Glenn: I was diagnosed with LQT through routine checkup at age "49". No family members that I know of had this but just found out that one uncle died around 45 no apparent reason, found dead in bed. I personally had no symptoms until 2 years ago. Got flu and dehydrated and started fainting. AFter recuperation, continued to faint and near faint for the rest of year even after beta blockers. Blood tests were all great. Went through couple of EP's and ended up at Rochester NY with Dr. Moss's group, a Dr Spencer Rosero who made the call for Medtronic ICD/pacemaker combination this past June. I have "carelink monitor" that I do from home and send over phone. I still have to see Dr. if problems or at least every 3-6 months in person. I am still on betas, hoping he will lower dose or remove since I have ICD. I do not have genetic results back...in research program at Rochester University in NY which takes much longer than commercial but it is free. Best of luck with Elizabeth, so glad you guys are so involved, she will do great. Have they told you when Elizabeth might be a candidate for ICD? We were torn up pretty bad by Hurricane Rita from current home to new home under construction to barns to storage sheds! But were are now in recovery mode, cannot believe it all happened. Thank God our family survived! We have lived here 35 years and never been affected by any storms, we live about 40 miles from coast, guess not far enough! Jmac (Judi) | ||
| Jmac |
Glenn: Where did you have Elizabeth's genetic testing done? | ||
| GMF |
Judi: Glad to hear that no additional known family members are in the LQT Club. Do you have children? Were they tested with EKGS and electrocardiograms? Liz's genetic tests were done by Genaissnace Pharmaceuticals. Although her test cost $5,400, the results were available within 8 weeks. We now have the LQT3 answer so we can treat accordingly and test other family members. It seems like the ICD is the best protective treatment for many LQT patients. Lowering your betas will be another ICD benefit. How has your adjustment been with the ICD? Liz had her pacemaker and medicines since her second day so these treatments will be a "normal" part in her game of life. LQT3 Liz is a good candidate for the ICD when she's older. I've been doing extensive research and found the Boston Children's Hospital has implanted ICDS in a few very young patients. Most of those cases are life or death, last-resort situations. Since Little Liz seems to be responding well with her growth and development, her doctor believes that she can wait. We'll feel safer when Liz finally gets an ICD. Until then, we have an AED and heart monitor for extra layers of protection. Thanks for the words of encouragement and well wishes for Little Liz. We are feeling more comfortable each day with Liz's LQT and looking forward to enjoying both of our children! Our goal in her life is that Liz isn't defined by LQT! I can already tell this little girl is always going to make daddy melt with her smiles. I'm sorry to hear that Hurricane Rita has caused havoc on your family's lives. Thankfully, you still have the most important valuables in life...your family! How is your family responding to the mental and physical damages in your lives? Best wishes for your family's recovery...rebuild, rediscover and re-energize the happiness and health in your lives! Have a wonderful weekend. Glenn FYI...check out the Long QT Group on Yahoo. http://health.groups.yahoo.com/group/longqt/ | ||
| Dr. Jorgen Kanters Username: Jorgen Registered: 08-2003 |
I must say that I dont agrre that ICD is the best treatment for many LQTS patients. I believe it is the best treatment for LQTS patients with an aborted cardiac arrest, and for LQTS patients having faintings despite betablocker treatment. It maybe a good choice in LQT3 patients since they not respond very well to betablockers. It is important to know that ICDs can occasionally kill patients too, so it is not a wonderful expensive solution. | ||
| GMF |
Yes, I miscommunicated my thoughts regarding ICD treatment. Since I'm not a doctor, all I know about LQT is only based on communicating with EPs and reading medical research. Maybe my statement should have been that ICDs are the treatments for some patients like you qualified in your response. I do know that my LQT3 daughter will probably be a candidate for an ICD when she's older. When you say that ICDs can occasionally kill, do you mean from the surgical procedure or malfunctioning ICD? I've read that ICDs are 99+% effective. What are some of the major problems that can occur with ICDs? I think it is essential for patients to evaluate both the pros and cons. What do you think about gene therapy for as a mean for LQT Treatment in the future...like 10-15 years? I'm just another LQT parent looking for every possible protection to keep optimistic for my daughter's happy and healthy life! Have a wonderful weekend. | ||
| Dr. Jorgen Kanters Username: Jorgen Registered: 08-2003 |
Both the procedure during the initial implantation, and later if the leads are going to be removed there is a substantial risk. Occasionally the ICD misinterpret a benign arrhythmia leading to a fatal arrhythmia (In LQTS it is not so common). Living with an ICD is not wonderful, and suicides are unfortunately seen. LQTS is dangerous but no so dangerous. We therefore have to select carefully the LQTS patients which are ICD candidates. LQT3 is a problem both because betablockers are not effective, but our knowledge is sparse since LQT3 is rare. Mexilitine is effective in shortening the QT interval in LQT3, but we dont know if prolongs life (or if actually shortens life). Only medical research studies could show that in the future. | ||
| Bionic Roadrunner |
Hello Glenn, May be I can tell you a bit about my personal experience with ICD's. Just 3 days ago, I received my 4th one. As Dr Kanters mentioned it, the risks during the implantation are there, but there are risks in any surgery and it is up to you to take those risks or not. Then there are risks of malfunction. I had one lead fractured with my first one after 4 years and I received a new device and a new lead. The old lead was left in and so were the "paddles" around my heart. This second ICD gave me no problem whatsoever and was replaced because the battery was getting low after 5 years. The second lead was left in place. The third one was another story. The second lead had to be removed and that was very tricky and created a lot of problems but the ICD saved my life many times because I had several episodes of LQT. It was just replaced because again the battery was low. The surgery went very well and the doctor even upgraded the device to a dual chamber and added a second lead that went very easily into the vein, next to the other one. So, I have old "hardware" that will stay there for ever because it would be dangerous to take them out since they have been there for so long. Now leads are removed with lasers and the procedure is easier and less dangerous. As you can see, everything improves. ICDs are much smaller, last longer, so do leads. there will always be risks, but you have to weigh them against the benefits. If an ICD is the last resort you do not have much choice. It was for me and I am glad I chose to get one, I would not be here to tell you about it otherwise. 15 years and 4 ICD later I am very healthy (except for a bit of an electrical problem in my heart  ), but my implanted paramedic shocks me and I pick up and I know i must pick up and go. It is not always easy, but I have a wonderful family, friends and doctors. You sound like a very caring father and I know your daughter would have the same kind of support I have. Keep on looking for answers. When I was first diagnosed, very little was known, but with wonderful doctors and researchers like Dr Kanters, we are learning more and more. The more you know the more power you will have, but do not let what you read scare you, which can happen sometimes. Remember that each case is different from the other and what happens to one patient does not necessarely happens to the other or all of them. All this to make you understand that the ICD are getting better all the time. Yes there are malfunctions but remember that they are made by human and humans err. There is no such thing as 100% perfect. So best wishes and good luck to your little girl! I hope that the solution you choose will be the best for her. | ||
| Bionic Roadrunner |
I should have added that there should be trials going on for a new defibrillator without intravenous leads, developped by Cameron Health. These new devices will probably not be availbale for years, but your little girl, might be of the generation that will benefit from them. | ||
| GMF |
I appreciate your in-depth feedback regarding your personal ICD experiences. I'm glad your last surgery went well. Best wishes for a healthy & lengthy time with your 4th ICD! I'm assuming that two replacements due to low batteries instead of technical difficulties is normal. Since batteries eventually drain, those replacements have to be expected. I'm curious what is the maximum number of ICD replacements for one of Dr. Kanter's patients? I completely understand there a numerous potential physical and psychological problems with ICDs. I know for some like yourself, there aren't alternatives and it's the last option. After learning about my daughter's LQT, I definitely have a new appreciation that nothing is 100%. I do comprehend that each LQT Case is unique so I've been advised by our EP to be careful with information that I read via the Internet. I liked your expression "I am very healthy (except for a bit of an electrical problem in my heart." I feel the same way about my daughter. Today, we use a pacemaker and medicines to keep her smiling. For now there is no choosing since the pacemaker was a necessity. One day, there will be an ICD Choice...we'll research and evaluate the latest technologies. The newest ICD approach at Children's Hospital Boston involves placing a standard transvenous lead in the pericardial space. Since my daughter already has 2 leads for her pacemaker, so I'm guessing her pacemaker leads have a similiar possibility to fracturing as ICD ones? Thanks for your well wishes for my daughter and compliments regarding my caring. I agree with your thoughts that knowledge is power. I just wish there was more existing LQT medical information and research so I'd feel more empowered! I'm sure yours and my hopes include medical and technological advancements so all the people with LQT have better opportunities. Do you know your LQT Type? Do other family members have LQT? My daughter has LQT3. We're currently genetic testing other members of my immediate family. Thanks again for your ICD insight! Best of Luck with your LQT Adventures! | ||
| Dr. Jorgen Kanters Username: Jorgen Registered: 08-2003 |
Normally an ICD lasts between 5 and 10 years on average. | ||
| Bionic Roadrunner |
Hello Glenn, I have LQT2 and one of my children has LQT2 also but has not had any problems so far (knocking on wood is required as you read this ). We were tested years ago as part of a research study, but my doctor had put her on betablockers before he obtained the results because she fell into the "borderline" category. Do not worry too much about the leads. Great progress has been made and I hear that the recent ones have less of a chance to break than the older ones. In fact they last longer than the defibrillators and can be reused from one to the other. My first defibrillator lasted 4 and a half years, my second one 5 years and the third one 5 and a half years. I made good use of the 3rd one by being shocked 15 times the last 2 years so it may have lasted longer... The surgeon gave it to my husband as a souvenir last week when I came out of surgery. Two days ago, we heard a strange beep coming from the spot where he had put it and we realized that it was the alarm signaling that the battery needed replacement! Talk about good timing. We had a good laugh about it. The psychological problems that can arise from living with an ICD can range from none to debilitating. I met an older man who had been shocked 47 times after a malfunction and never slept in his pyjamas, but all dressed up in his regular clothes with a suitcase packed in case they had to take him to the hospital. I had multiple shocks too and they are difficult to deal with, but with good help and support I manage to deal with it. There are studies being done right now on how to deal with the shocks and fear of them. I am not sure sure that all patients get enough information about what to expect of their ICD and this is why it is good to join a support group if at all possible. Hopefully, the studies will lead to more knowledge of these effects and psychologist will be able to help patients from day one, without waiting for the problems to arise. I think you are doing everything possible to educate yourself and help your little girl. I believe like for anyone of us, the rest is in the hands of higher powers... Good luck again to you and your family. Keep in touch and let us know how things go. PS Have you and other members of your family been tested? | ||
| GMF |
That's funny about your old ICD beeping for a low battery. A friend told me that her husband's low battery beeping began during a moment of intimacy. Pretty funny and scary if you don't know what the beeping means. Thanks for your encouragement regarding my LQT and ICD pursuit of information. It's certainly helpful to hear the personal accounts from those living the ICD Adventure. I'm already exploring the psychological aspect of ICDs. Hopefully my wife's training and experience as a clinical psychologist will help ease my daughter's mental challenges. Her former mentors recently switched their psychology research to heart / ICD patients. Since psychological problems are possible, I agree that support groups can provide valuable help and information. I've already gained a lot of LQT knowledge from this website and others. We're waiting for genetic test results for our immediate family (wife, son & me). We'll hope that my daughter is the only one in the LQT Club. Whatever happens, we'll know the answers. Some extended family members had their children given an EKG. |